ABCD4 Antibody

CSB-PA047241

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Size:

50μl100μl

US$166

Quantity:

Species Reactivity: Human,Mouse

Raised in: Rabbit

Application: ELISA,WB

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Target Background

Product Details

Uniprot NO.

O14678

Target Names

ABCD4

Alternative Names

ABCD4; PXMP1L; Lysosomal cobalamin transporter ABCD4; ATP-binding cassette sub-family D member 4; PMP70-related protein; P70R; Peroxisomal membrane protein 1-like; PXMP1-L; Peroxisomal membrane protein 69; PMP69

Raised in

Rabbit

Species Reactivity

Human,Mouse

Immunogen

Synthetic peptide of Human ABCD4

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Isotype

IgG

Purification Method

Antigen affinity purification

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Tested Applications

ELISA,WB

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Protocols

ELISA Protocol Western Blotting(WB) Protocol

Datasheet & COA

Antibody FAQs

Images

Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Jurkat cells, Primary antibody: CSB-PA047241(ABCD4 Antibody) at dilution 1/700, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 minute

Description

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Target Background

Function

Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner. Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane. Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane.

Gene References into Functions

endogenous ABCD4 was localized to both lysosomes and the ER, and its lysosomal localization was disturbed by knockout of LMBRD1 PMID:27456980
Data suggest that ABCD4 lysosomal targeting depends on co-expression of and interaction with LMBRD1; mutations in LMBRD1 and ABCD4 that result in cobalamin metabolism disorders cblF and cblJ (or mutations in ATPase domain) disrupt interactions between LMBRD1 and ABCD4. (LMBRD1 = nuclear export signal-interacting protein; ABCD4 = ATP-binding cassette, sub-family D (ALD), member 4) PMID:28572511
Results propose a model whereby membrane-bound LMBD1 and ABCD4 facilitate the vectorial delivery of lysosomal vitamin B12 to cytoplasmic MMACHC. PMID:25535791
mutations altering the putative ATPase domain of ABCD4 affect its function, suggesting that the ATPase activity of ABCD4 may be involved in intracellular processing of vitamin B12. PMID:22922874
Defects in ABCB4 have been found to cause progressive familial intrahepatic cholestasis type 3. PMID:21514256
expression tends to be correlated with the severity of X-linked adrenoleukodystrophy PMID:15800013